PKP2 and Arrhythmogenic right ventricular dysplasia: Furthermore, in a recently published case-control analysis of DCM genes, PKP2 and PDLIM3 variants were not enriched in cases compared with a control population.26 Whereas PKP2 has previously been curated as a definitive-evidence gene for the ARVC phenotype with several rare, predicted loss-of-function variants published in ARVC cases,35 when the clinical data for a strict DCM phenotype were curated, the currently available literature provided only a few missense variants and was considered insufficient.